CHAPTER 12 AUTOIMMUNE BLISTERING DISEASES 189
Pemphigoid Complex
Heterogenous group of autoantibodies targeting various basement membrane zone
components.
Targets include specific (αβ) integrins, plakins (bullous pemphigoid antigen), and
collagen type XVII.
Autoantibodies in mucous membrane pemphigoid may also target laminin 5.
Binding of autoantibodies at the BMZ leads to activation of complement and recruit-
ment of inflammatory cells; released proteases damage the BMZ to cause blister
formation.Epidermolysis Bullosa Acquisita
Autoantibodies target collagen VII within hemidesmosomes.
Pathomechanism similar to pemphigoid: binding of autoantibodies at the BMZ leads
to activation of complement and recruitment of inflammatory cells; released proteases
damage the BMZ to cause blister formation.SIGNALMENT/HISTORY
Pemphigus Complex
Uncommon group of diseases.
Usually middle-aged to old animals.
PF:
Most common type
Dogs: Akita, chow chow, dachshund, spaniels, English bulldog; median age of
onset 4 years; rarely develops in young dogs.
Cats: no breed or sex predisposition; median age of onset 5 years.
Most commonly implicated drug causes: antibiotics (sulfonamides, cephalexin),methimazole.
Insecticide-contact PF: amitraz-metaflumizone product; large-breed dogs(Figure 12.1).
PE:
Uncommon
May be a more benign variant of PF or may be a cross-over syndrome of pem-
phigus and lupus erythematosus
Collie, German shepherd dog, and Shetland sheepdog
Aggravated by ultraviolet light exposure. PV:
Rare
Most severe form
German shepherd dog, collie; males predisposed; median age of onset 6 years.
PEP/Pveg: rarest type; course of disease may represent a more severe variant of PF.
PP: rare; clinical signs vary from severe to relatively benign crusted lesions.